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When Your Child Has Aortic Stenosis (AS)

The heart is divided into 4 chambers. The 2 upper chambers are called atria and the 2 lower chambers are called ventricles. The heart contains 4 valves. The valves open and close to keep blood flowing forward through the heart. The aortic valve is located between the left ventricle and the aorta. It has three leaflets that open and close to allow blood through. It controls the flow of blood from the heart to the rest of the body (except for the lungs). Aortic stenosis (AS) occurs when this valve doesn’t open all the way. It can also occur when the area above or below the valve is too narrow. As a result, blood flow from the heart to the rest of the body is obstructed (blocked). Untreated, this condition can lead to certain heart problems over time. ﻿But good treatments are usually available.

Inside view of a normal heart showing the left heart structures

Top view of heart

Types of Aortic Stenosis

An open normal aortic valve (view from above)

An open aortic valve with stenosis

AS can be described as:

Supravalvar, when obstruction occurs above the valve (the aorta may be too narrow).

Valvar, when obstruction occurs at the valve (leaflets may be too thick or are stuck together).

Subvalvar, when obstruction occurs below the valve (area below the valve may be too narrow).

What Causes Aortic Stenosis?

AS is a congenital heart defect. This means it’s a problem with the structure of the heart that your child was born with. It can be the only defect, or it can be part of a more complex set of defects. The exact cause is unknown, but most cases seem to occur by chance. Having a family history of left-sided heart defects can be a risk factor.

Why Is Aortic Stenosis a Problem?

AS forces the left ventricle to work harder to pump blood through the aortic valve into the aorta to reach the body. This causes the left ventricle to thicken (hypertrophy) and get larger. Over time, the left ventricle can become so overworked that it no longer pumps blood well. This condition is known as congestive heart failure (CHF).

Children with valve problems such as AS may be at risk of developing an infection of the heart’s inner lining or valves. This infection is called infective endocarditis.

What Are the Symptoms of Aortic Stenosis?

Children with mild or moderate AS can appear to be in normal health and have no symptoms. Children with severe or critical AS will usually have symptoms. These can include:

Dizziness and fainting

Trouble breathing or rapid breathing

Chest pain

Trouble feeding (in infants)

Tiring easily during exercise (in older children)

How Is Aortic Stenosis Diagnosed?

During a physical exam, the doctor checks for signs of a heart problem such as a heart murmur. This is an extra noise caused when blood doesn’t flow smoothly through the heart. If a heart problem is suspected, your child will be referred to a pediatric cardiologist. This is a doctor who diagnoses and treats heart problems in children. To check for AS, the following tests may be done:

Chest X-ray. X-rays are used to take a picture of the heart and lungs.

﻿﻿Electrocardiogram (ECG or EKG). The electrical activity of the heart is recorded.

Echocardiogram (echo). Sound waves (ultrasound) are used to create pictures of the heart and look for structural defects.

How Is Aortic Stenosis Treated?

After treatment for aortic stenosis, your child can be active.

Mild or moderate AS usually requires no treatment. But regular visits with a cardiologist are needed. This is to make sure that narrowing at or near the valve doesn’t worsen over time.

Severe or critical AS requires treatment. Medications may be prescribed to help relieve symptoms. Further treatment will be needed to relieve the obstruction to blood flow. The valve can be opened with a procedure called balloon valvuloplasty. Or, the valve can be repaired or replaced with heart surgery. Your child’s condition will be evaluated and the cardiologist will discuss treatment options with you.

﻿ After treatment for aortic stenosis, your child can be active.

What Are the Long-term Concerns?

All treatment options for AS are palliative (relieve symptoms). This means that the aortic valve is not repaired and will always be abnormal. Further problems with the valve may occur again in the future.

After treatment, most children with AS can be active. The level and extent of physical activity will vary with each child. Certain contact sports, such as football, may need to be avoided. Check with the cardiologist about what activities are appropriate for your child.

Regular follow-up visits with the cardiologist will be needed for the rest of your child’s life. This is to make sure the valve doesn’t become obstructed again or have too much leakage. Children with valve replacements are more likely to need further surgery in the future.

If Your Child Has a Bicuspid Aortic Valve

A bicuspid aortic valve has two leaflets instead of three. The leaflet tissue may also be abnormally thick. This defect may or may not cause AS, so symptoms aren’t always obvious. It can often go undetected into adulthood. If the problem is diagnosed in childhood, treatment is only needed if the valve obstruction worsens over time, or if the part of the aorta above the valve (aortic root) becomes too dilated, or enlarged. A child with a bicuspid aortic valve may be at higher risk of infective endocarditis. Even if the diagnosis is not made until adulthood, the valve may require replacement later in life.